Complement activation in renal disease.

Abstract

Perturbation of the serum complement system in glomerulonephritis can be the result of classical pathway activation by immune complexes or of C3 activation by the C3-C5 convertase stabilized by the C3 nephritic factor. Low C3 levels can also be the result of diminished C3 synthesis and possibly, in certain circumstances, of C3 convertases deposited on capillary walls. In glomerulonephritis the complement profile is helpful in diagnosis, in following the course of therapy and in providing insights into pathogenesis. Complement profiles must be interpreted recognizing that a pattern resembling classical pathway activation can be produced by idiopathic nephrotic syndrome, that hypogammaglobulinemia can reduce Clq levels, and that a primary deficiency of factor H or I or both, will secondarily produce subnormal levels of C3 and factor B. With these caveats, the complement profiles typical of systemic lupus erythematosus, membranoproliferative glomerulonephritis, acute glomerulonephritis, acquired Cl inhibitor deficiency, and hypocomplementemic vasculitis syndrome are described.